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Neuropathological Characterisation of McLeod Syndrome With a Proposed New Grading System
Neuropathology and Applied Neurobiology, Volume 51, Issue 5, October 2025.We report the neuropathological findings of the X‐linked McLeod neuroacanthocytosis syndrome (MLS), a very rare neurodegenerative disorder characterised by red blood cell acanthocytosis, chorea syndrome similar to Huntington’s disease, and additional neuromuscular and cardiological involvement.
Anna Maria Reuss +11 more
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UEG Week 2025 Poster Presentations
United European Gastroenterology Journal, Volume 13, Issue S8, Page S803-S1476, October 2025.
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Superantigens in demyelinating disease
Springer Seminars in Immunopathology, 1996Autoreactive T cells are part of the normal lymphocyte repertoire of healthy individuals [4]. In the immune system of healthy individuals, potent regulatory mechanisms control T and B cell tolerance to self antigens. While genetic factors influence susceptibility to autoimmunity in humans and experimental animals, environmental factors contribute to ...
Stefan Brocke +2 more
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CNS demyelination in autoimmune diseases [PDF]
Clinical Neurology and Neurosurgery, 2006 Autoimmune diseases represent a diverse group of disorders that have generally of unknown etiology and poorly understood pathogenesis. They may be organ-specific or systemic, giving rise to overlapping syndromes; more than one autoimmune disease may occur in the same patient.
Davorka Vranješ +5 more
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Autoimmunity in Demyelinating Diseases
Annals of the New York Academy of Sciences, 1987Demyelinative diseases of the CNS and peripheral nervous system can be distinguished on the basis of primary mediation by antibody or T lymphocytes (or failure of the T-cell-mediated response) and on the basis of chronicity. The principal mechanisms are autoimmunization to myelin antigens after actual immunization with tissue or infection with cross ...
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Pathology of Demyelinating Diseases
Annual Review of Pathology: Mechanisms of Disease, 2012There has been significant progress in our understanding of the pathology and pathogenesis of central nervous system inflammatory demyelinating diseases. Neuropathological studies have provided fundamental new insights into the pathogenesis of these disorders and have led to major advances in our understanding of multiple sclerosis (MS) heterogeneity ...
Claudia F. Lucchinetti +1 more
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Demyelinating diseases in Asia
Current Opinion in Neurology, 2016The present review aims to discuss the recent advances in inflammatory demyelinating diseases of the central nervous system in Asia.Prevalence of multiple sclerosis (MS) in Asia is lower than that in Western countries, although it has been increasing recently.
Kazuo Fujihara, Hirofumi Ochi
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Pediatric Demyelinating Diseases
Continuum, 2013In the past decade, the number of studies related to demyelinating diseases in children has exponentially increased. Demyelinating disease in children may be monophasic or chronic. Typical monophasic disorders in children are acute disseminated encephalomyelitis and clinically isolated syndromes, including optic neuritis and transverse myelitis ...
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Demyelination in peroxisomal diseases
Journal of the Neurological Sciences, 2005Peroxisomal disorders that display neurologic involvement usually show a variety of abnormalities in white matter of the central nervous system (CNS). Adult Refsum’s disease primarily exhibits a hypertrophic (onion bulb) demyelinating neuropathy. The changes in CNS white matter vary greatly between these diseases, but basically can be divided into ...
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The Biochemistry of Demyelination and Demyelinating Diseases
1972Wallerian degeneration was the first experimental model of demyelination devised and has been widely investigated both structurally and biochemically. After severing a peripheral nerve there is increasing loss of cerebrosides, sphingomyelin, cholesterol and phospholipids, that of the cephalins starting earlier and exceeding that of lecithin [1]. During
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